Idiopathic pulmonary fibrosis differential diagnosis
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Differentiating Idiopathic pulmonary fibrosis from other Diseases |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Idiopathic pulmonary fibrosis must be differentiated from other causes of interstitial lung diseases which may cause fibrosis as well. Other diseases may include acute interstitial pneumonia, hypersensitivity pneumonia, occupational lung diseases, and pulmonary hemorrhage diseases.
Differentiating Interstitial Lung Disease from other Diseases
To review the complete differential diagnosis of dyspnea, click here.
To review the complete differential diagnosis of hemoptysis, click here.
To review the complete differential diagnosis of restrictive lung disease, click here.
Abbreviations: ABG: Arterial blood gas, BAL: Bronchoalveolar lavage, ESR: Erythrocyte sedimentation rate, CRP: C–reactive protein, FVC: Forced vital capacity, RV: Residual volume, FEV1: Forced expiratory volume during the 1st second, DLCO: Diffusing capacity of the lungs for carbon monoxide, O2: Oxygen, TLC: Total lung capacity, PaO2: Arterial partial pressure of oxygen, FiO2: Fraction of inspired oxygen, LDH: Lactate dehydrogenase, CEA: Carcinoembryonic antigen, Anti-GBM antibody: Anti-glomerular basement membrane antibody, A−a gradient: Alveolar-arterial gradient, PAS: Periodic acid-Schiff stain, LAM: Lymphangiomyomatosis, IgE: Immunoglobulin E, ANCA: Anti-neutrophil cytoplasmic antibody, RBC: Red blood cell, ACE: Angiotensin-converting enzyme
Disease | Clinical manifestation | Investigations | ||||||||||||||||||||
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History | Symptoms | Physical examination | Lab findings | Imaging | Pulmonary function test | Bronchoscopy and BAL | Gold standard | |||||||||||||||
Duration | Age | Gender | Family history | Smoking history | Environmental exposure | HIV | Dyspnea | Cough | Wheezing | Chest pain | Tachypnea | Auscultation | Cyanosis | Clubbing | Spirometry | ABG | ||||||
Idiopathic pulmonary fibrosis[1] | Chronic | 60−70 years old | Men | + | + | ± | − | + | Dry | + | + | + | − | + |
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Idiopathic nonspecific interstitial pneumonia[2] | Acute/Chronic | 50−60 years old | Female | + | − | − | + | + | + | + | − | + |
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− | ± |
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Cryptogenic organising pneumonia[3] | Acute/subacute | 50−60 years old | Both | − | ± | − | − | + | Dry | − | − | − |
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− | − |
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Disease | History | Symptoms | Physical examination | Lab findings | Imaging | Pulmonary function test | Bronchoscopy and BAL | Gold standard | ||||||||||||||
Duration | Age | Gender | Family history | Smoking history | Environmental exposure | HIV | Dyspnea | Cough | Wheezing | Chest pain | Tachypnea | Auscultation | Cyanosis | Clubbing | Spirometry | ABG | ||||||
Acute interstitial pneumonia (Hamman−Rich syndrome)[4] | Acute | 50−60 years old | Both | − | − | − | − | + | + | − | − | + |
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− | − |
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Lymphocytic interstitial pneumonia[5] | Subacute | 30−40 years old | Female | − | − | − | ± | + | + | + | + | − |
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− | + |
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| ||||
Respiratory bronchiolitis−interstitial lung disease[6] | Subacute | 30−40 years old | Both | − | + | − | − | + | Dry | + | − | − | − | − |
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Desquamative interstitial pneumonia[7][8] | Chronic | 40−50 years old | Both | − | + | − | − | + | Dry | + | − | − |
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− | − |
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Disease | History | Symptoms | Physical examination | Lab findings | Imaging | Pulmonary function test | Bronchoscopy and BAL | Gold standard | ||||||||||||||
Duration | Age | Gender | Family history | Smoking history | Environmental exposure | HIV | Dyspnea | Cough | Wheezing | Chest pain | Tachypnea | Auscultation | Cyanosis | Clubbing | Spirometry | ABG | ||||||
Pulmonary Langerhans cell granulomatosis[9] | Chronic | 20−40 years old | Both | + | + | − | − | ± | Dry | + | + | − |
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− | − |
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Pulmonary alveolar proteinosis[10][11] | Acute/chronic | 40−50 years old | Male | + | + | + | − | + | + | + | − | − |
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+ | + |
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Pulmonary lymphangioleiomyomatosis[12] | Acute/chronic | 30−40 years old | Female | + | + | − | − | + | Bloody | + | + | − |
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− | + |
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Eosinophilic pneumonia[13] | Acute/chronic | 20−40 years old | Male | − | − | − | − | + | Dry | + | + | + |
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− | − |
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Hypersensitivity pneumonitis[14] | Acute/subacute/chronic | 40−60 years old | Both | − | ± | + | − | + | Dry/productive | + | + | + |
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− | + |
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Disease | History | Symptoms | Physical examination | Lab findings | Imaging | Pulmonary function test | Bronchoscopy and BAL | Gold standard | ||||||||||||||
Duration | Age | Gender | Family history | Smoking history | Environmental exposure | HIV | Dyspnea | Cough | Wheezing | Chest pain | Tachypnea | Auscultation | Cyanosis | Clubbing | Spirometry | ABG | ||||||
Occupational lung disease[15] | Chronic | Elderly | Male | + | + | + | − | ± | + | + | + | + |
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Peripheral/central | + |
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| |
Radiation−induced lung injury[16] | Subacute/chronic | Any age | Both | − | − | + | − | + | Dry | + | + | + |
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+ | − |
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Pulmonary hemorrhage syndromes | Goodpasture syndrome[17] | Chronic | All ages | Male | + | ± | − | − | ± | Bloody | ± | − | − |
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− | − |
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Idiopathic pulmonary hemosiderosis[18] | Acute/subacute/chronic | Children − 10 years old | Both | + | ± | − | − | + | Bloody | + | + | − | − | − |
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Isolated pulmonary capillaritis[19] | Chronic | 40−60 years old | Both | + | − | ± | − | + | Bloody | + | + | + |
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− | − |
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Disease | History | Symptoms | Physical examination | Lab findings | Imaging | Pulmonary function test | Bronchoscopy and BAL | Gold standard | ||||||||||||||
Duration | Age | Gender | Family history | Smoking history | Environmental exposure | HIV | Dyspnea | Cough | Wheezing | Chest pain | Tachypnea | Auscultation | Cyanosis | Clubbing | Spirometry | ABG | ||||||
Sarcoidosis[20] | Acute/subacute/chronic | 20−40 years old | Female | + | ± | − | − | ± | + | + | ± | − |
|
+ | − |
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|
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| |||
Granulomatous vasculitides | Granulomatosis with polyangiitis (Wegener)[21] | Chronic | Elderly | Both | + | − | − | − | + | + | + | ± | − | − | − |
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| |
Eosinophilic granulomatosis with polyangiitis (Churg Strauss)[22] | Chronic | 40−50 years old | Both | + | − | − | − | − | + | + | − | − |
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− | − |
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Bronchocentric granulomatosis[23] | Chronic | 30−70 years old | Both | − | − | − | − | ± | ± | + | ± | − |
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− | − |
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Pulmonary lymphomatoid granulomatosis[24] | Chronic | 30−50 years old | Male | − | − | − | − | + | + | + | + | − |
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− | − |
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Amyloidosis[25][26] | Subacute/chronic | 50−70 years old | Male | + | − | − | − | − | Bloody | + | − | − | − | − |
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Disease | History | Symptoms | Physical examination | Lab findings | Imaging | Pulmonary function test | Bronchoscopy and BAL | Gold standard | ||||||||||||||
Duration | Age | Gender | Family history | Smoking history | Environmental exposure | HIV | Dyspnea | Cough | Wheezing | Chest pain | Tachypnea | Auscultation | Cyanosis | Clubbing | Spirometry | ABG |
References
- ↑ Poletti, Venerino; Ravaglia, Claudia; Buccioli, Matteo; Tantalocco, Paola; Piciucchi, Sara; Dubini, Alessandra; Carloni, Angelo; Chilosi, Marco; Tomassetti, Sara (2013). "Idiopathic Pulmonary Fibrosis: Diagnosis and Prognostic Evaluation". Respiration. 86 (1): 5–12. doi:10.1159/000353580. ISSN 1423-0356.
- ↑ Travis, William D.; Hunninghake, Gary; King, Talmadge E.; Lynch, David A.; Colby, Thomas V.; Galvin, Jeffrey R.; Brown, Kevin K.; Chung, Man Pyo; Cordier, Jean-François; du Bois, Roland M.; Flaherty, Kevin R.; Franks, Teri J.; Hansell, David M.; Hartman, Thomas E.; Kazerooni, Ella A.; Kim, Dong Soon; Kitaichi, Masanori; Koyama, Takashi; Martinez, Fernando J.; Nagai, Sonoko; Midthun, David E.; Müller, Nestor L.; Nicholson, Andrew G.; Raghu, Ganesh; Selman, Moisés; Wells, Athol (2008). "Idiopathic Nonspecific Interstitial Pneumonia". American Journal of Respiratory and Critical Care Medicine. 177 (12): 1338–1347. doi:10.1164/rccm.200611-1685OC. ISSN 1073-449X.
- ↑ Mehrian, P.; Doroudinia, A.; Rashti, A.; Aloosh, O.; Dorudinia, A. (2017). "High-resolution computed tomography findings in chronic eosinophilic vs. cryptogenic organising pneumonia". The International Journal of Tuberculosis and Lung Disease. 21 (11): 1181–1186. doi:10.5588/ijtld.16.0723. ISSN 1027-3719.
- ↑ Parambil, Joseph; Mukhopadhyay, Sanjay (2012). "Acute Interstitial Pneumonia (AIP): Relationship to Hamman-Rich Syndrome, Diffuse Alveolar Damage (DAD), and Acute Respiratory Distress Syndrome (ARDS)". Seminars in Respiratory and Critical Care Medicine. 33 (05): 476–485. doi:10.1055/s-0032-1325158. ISSN 1069-3424.
- ↑ Panchabhai, Tanmay S.; Farver, Carol; Highland, Kristin B. (2016). "Lymphocytic Interstitial Pneumonia". Clinics in Chest Medicine. 37 (3): 463–474. doi:10.1016/j.ccm.2016.04.009. ISSN 0272-5231.
- ↑ Sieminska, Alicja; Kuziemski, Krzysztof (2014). "Respiratory bronchiolitis-interstitial lung disease". Orphanet Journal of Rare Diseases. 9 (1). doi:10.1186/s13023-014-0106-8. ISSN 1750-1172.
- ↑ Ryu, Jay H.; Myers, Jeffrey L.; Capizzi, Stephen A.; Douglas, William W.; Vassallo, Robert; Decker, Paul A. (2005). "Desquamative Interstitial Pneumonia and Respiratory Bronchiolitis-Associated Interstitial Lung Disease". Chest. 127 (1): 178–184. doi:10.1378/chest.127.1.178. ISSN 0012-3692.
- ↑ Craig, P J; Wells, A U; Doffman, S; Rassl, D; Colby, T V; Hansell, D M; du Bois, R M; Nicholson, A G (2004). "Desquamative interstitial pneumonia, respiratory bronchiolitis and their relationship to smoking". Histopathology. 45 (3): 275–282. doi:10.1111/j.1365-2559.2004.01921.x. ISSN 0309-0167.
- ↑ Blakley, Matthew P.; Dutcher, Janice P.; Wiernik, Peter H. (2018). "Pulmonary Langerhans cell histiocytosis, acute myeloid leukemia, and myelofibrosis in a large family and review of the literature". Leukemia Research. 67: 39–44. doi:10.1016/j.leukres.2018.01.011. ISSN 0145-2126.
- ↑ Carrington JM, Hershberger DM. PMID 29493933. Missing or empty
|title=
(help) - ↑ Kiani, Arda; Parsa, Tahereh; Adimi Naghan, Parisa; Dutau, Hervé; Razavi, Fatemeh; Farzanegan, Behrooz; Pourabdollah Tootkaboni, Mahsa; Abedini, Atefeh (2018). "An eleven-year retrospective cross-sectional study on pulmonary alveolar proteinosis". Advances in Respiratory Medicine. 86 (1): 7–12. doi:10.5603/ARM.2018.0003. ISSN 2543-6031.
- ↑ Xu, Kai-Feng; Lo, Bee Hong (2014). "Lymphangioleiomyomatosis: differential diagnosis and optimal management". Therapeutics and Clinical Risk Management: 691. doi:10.2147/TCRM.S50784. ISSN 1178-203X.
- ↑ Bernheim, Adam; McLoud, Theresa (2017). "A Review of Clinical and Imaging Findings in Eosinophilic Lung Diseases". American Journal of Roentgenology. 208 (5): 1002–1010. doi:10.2214/AJR.16.17315. ISSN 0361-803X.
- ↑ Miller, Ross; Allen, Timothy Craig; Barrios, Roberto J.; Beasley, Mary Beth; Burke, Louise; Cagle, Philip T.; Capelozzi, Vera Luiza; Ge, Yimin; Hariri, Lida P.; Kerr, Keith M.; Khoor, Andras; Larsen, Brandon T.; Mark, Eugene J.; Matsubara, Osamu; Mehrad, Mitra; Mino-Kenudson, Mari; Raparia, Kirtee; Roden, Anja Christiane; Russell, Prudence; Schneider, Frank; Sholl, Lynette M.; Smith, Maxwell Lawrence (2018). "Hypersensitivity Pneumonitis A Perspective From Members of the Pulmonary Pathology Society". Archives of Pathology & Laboratory Medicine. 142 (1): 120–126. doi:10.5858/arpa.2017-0138-SA. ISSN 0003-9985.
- ↑ Sirajuddin, Arlene; Kanne, Jeffrey P. (2009). "Occupational Lung Disease". Journal of Thoracic Imaging. 24 (4): 310–320. doi:10.1097/RTI.0b013e3181c1a9b3. ISSN 0883-5993.
- ↑ Giridhar P, Mallick S, Rath GK, Julka PK (2015). "Radiation induced lung injury: prediction, assessment and management". Asian Pac. J. Cancer Prev. 16 (7): 2613–7. PMID 25854336.
- ↑ DeVrieze BW, Hurley JA. PMID 29083697. Missing or empty
|title=
(help) - ↑ Khorashadi, L.; Wu, C.C.; Betancourt, S.L.; Carter, B.W. (2015). "Idiopathic pulmonary haemosiderosis: spectrum of thoracic imaging findings in the adult patient". Clinical Radiology. 70 (5): 459–465. doi:10.1016/j.crad.2014.11.007. ISSN 0009-9260.
- ↑ Thompson, Gwen; Klecka, Mary; Roden, Anja C.; Specks, Ulrich; Cartin-Ceba, Rodrigo (2016). "Biopsy-proven pulmonary capillaritis: A retrospective study of aetiologies including an in-depth look at isolated pulmonary capillaritis". Respirology. 21 (4): 734–738. doi:10.1111/resp.12738. ISSN 1323-7799.
- ↑ Li, Cheng-Wei; Tao, Ru-Jia; Zou, Dan-Feng; Li, Man-Hui; Xu, Xin; Cao, Wei-Jun (2018). "Pulmonary sarcoidosis with and without extrapulmonary involvement: a cross-sectional and observational study in China". BMJ Open. 8 (2): e018865. doi:10.1136/bmjopen-2017-018865. ISSN 2044-6055.
- ↑ Greco A, Marinelli C, Fusconi M, Macri GF, Gallo A, De Virgilio A, Zambetti G, de Vincentiis M (June 2016). "Clinic manifestations in granulomatosis with polyangiitis". Int J Immunopathol Pharmacol. 29 (2): 151–9. doi:10.1177/0394632015617063. PMC 5806708. PMID 26684637.
- ↑ Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, Altissimi G, De Vincentiis M (April 2015). "Churg-Strauss syndrome". Autoimmun Rev. 14 (4): 341–8. doi:10.1016/j.autrev.2014.12.004. PMID 25500434.
- ↑ Myers, Jeffrey L. (1989). "Bronchocentric Granulomatosis". Chest. 96 (1): 3–4. doi:10.1378/chest.96.1.3. ISSN 0012-3692.
- ↑ Ankita, Grover; Shashi, Dhawan (2016). "Pulmonary Lymphomatoid Granulomatosis- a Case Report with Review of Literature". Indian Journal of Surgical Oncology. 7 (4): 484–487. doi:10.1007/s13193-016-0525-1. ISSN 0975-7651.
- ↑ Khoor, Andras; Colby, Thomas V. (2017). "Amyloidosis of the Lung". Archives of Pathology & Laboratory Medicine. 141 (2): 247–254. doi:10.5858/arpa.2016-0102-RA. ISSN 0003-9985.
- ↑ Milani, Paolo; Basset, Marco; Russo, Francesca; Foli, Andrea; Palladini, Giovanni; Merlini, Giampaolo (2017). "The lung in amyloidosis". European Respiratory Review. 26 (145): 170046. doi:10.1183/16000617.0046-2017. ISSN 0905-9180.
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